Concordant optic glioma in a pair of monozygotic twins with neurofibromatosis.
The occurence is reported of optic chiasma gliomas in a pair of identical twins, both with neurofibromatosis. One twin developed precocious puberty in the sixth year of life and was shown by cisternal computerized tomography to have a supersellar tumor, which proved to be a malignant glioma. The second twin had an abnormal growth spurt but not overt precocious puberty. Cisternal computerized tomography of the second twin revealed evidence of a similar, smaller tumor at the same site. The literature is reviewed and some possible genetic explanations discussed. This report also emphasises the importance of careful investigation of the second twin when a malignancy is found in one, irrespective of the site or type of the tumor.
D002675:Child, Preschool; D003390:Cranial Nerve Neoplasms / Q000150:complications* / Q000000981:diagnostic imaging; D004200:Diseases in Twins; D005260:Female; D005910:Glioma / Q000150:complications* / Q000000981:diagnostic imaging; D006801:Humans; D008297:Male; D009456:Neurofibromatosis 1 / Q000150:complications*; D009897:Optic Chiasm; D011247:Pregnancy; D011629:Puberty, Precocious / Q000209:etiology; D011859:Radiography; D012878:Skin Neoplasms / Q000150:complications*; D014427:Twins; D014430:Twins, Monozygotic